Edit concept Question Editor Create issue ticket

Follicular Dendritic Cell Sarcoma


Presentation

  • Most commonly, FDC sarcoma presents as a solitary mass in lymph nodes, however, several extra-nodal locations have been identified.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: A case of FDCS in a 63-year-old woman, arising in the urinary bladder, a previously unreported site, is described.[ncbi.nlm.nih.gov]
  • A 67-year-old woman presented with a hypoechoic mass between the spleen and left kidney during a medical examination. The patient was hospitalized for further diagnosis. Her laboratory results did not present any obvious abnormal changes.[ncbi.nlm.nih.gov]
  • OBJECTIVE: Follicular dendritic cell sarcoma is a rare disease with a non-specific and insidious presentation that is further complicated by difficult diagnostic and therapeutic assessment.[ncbi.nlm.nih.gov]
  • Among the three cases, two cases are presented as retroperitoneal mass and one as mediastinal mass. CT scans revealed heterogeneously enhancing masses.[ncbi.nlm.nih.gov]
Soft Tissue Mass
  • Multidetector CT scan of the abdomen showed a 14-cm soft-tissue mass with calcification and necrosis within the greater omentum.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: A huge mediastinal shallowly-lobulated, demarcated soft tissue mass, with speckled, strip-like, coarse or arborizing calcification inside, and mild to moderate enhancement after contrast material administration on CT image, should consider[ncbi.nlm.nih.gov]
Fishing
  • MDM2 stained less than 5% to 20% of the tumor cells in 5 (36%) of 14 cases; 2 of them showed amplification by fluorescence in situ hybridization (FISH). CDK4 was negative except for weak staining in 1 of 14 cases.[ncbi.nlm.nih.gov]
  • Within the nodule, there were several hemorrhaged areas with a solid fish-flesh-like section. Representative hematoxylin and eosin staining of postoperative pathological examination showed: 1. Inflammatory pseudotumor-type (a) (b) Figure 1.[scirp.org]
Upper Abdominal Pain
  • We presented a 37-year-old woman who has suffered from obstructive jaundice, weight loss and right upper abdominal pain for 2 months.[ncbi.nlm.nih.gov]
  • PATIENT CONCERNS: A 19-year-old female presented with recent onset of left upper abdominal pain.[ncbi.nlm.nih.gov]
Jaundice
  • METHODS: We report a case of a 53-year-old female who presented with symptoms of abdominal pain, fever, anemia, and jaundice. After an extensive review of the literature, we have found only 12 cases of hepatic FDC sarcoma.[ncbi.nlm.nih.gov]
  • We presented a 37-year-old woman who has suffered from obstructive jaundice, weight loss and right upper abdominal pain for 2 months.[ncbi.nlm.nih.gov]

Treatment

  • The optimal treatment was undetermined.[ncbi.nlm.nih.gov]
  • Treatment recommendations are currently based on case reports and small series describing combinations of surgery, chemotherapy and radiotherapy providing the best patient outcomes.[ncbi.nlm.nih.gov]
  • We briefly discuss clinical history, histopathological differential diagnosis and treatment options of FDC sarcoma.[ncbi.nlm.nih.gov]
  • The initial diagnosis was lymphoma, and appropriate treatment was prescribed. After the initial treatment failed, additional biopsy samples were obtained.[ncbi.nlm.nih.gov]
  • To increase the understanding of this rare disease, we report our experience of three cases of FDCS with an analysis of the morphological and immunophenotypic characteristics, clinical course, treatment options and response to treatment.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis tends to be worse for patients with large or high-grade tumors and/or an intra-abdominal tumor site. For optimal management, complete surgical excision is recommended.[ncbi.nlm.nih.gov]
  • Very little is known about possible treatment options and overall prognosis. This case reports a 66 year-old patient, who underwent surgical removal of a persistently enlarged right cervical lymph node.[ncbi.nlm.nih.gov]
  • Also, so far we have found that the malignant degree of all patients diagnosed with malignant tumors have been reported were low and after combined treatment of surgery, radiotherapy and chemotherapy, most of the prognosis was good.[ncbi.nlm.nih.gov]
  • In this review, we discuss the clinicopathologic features of follicular dendritic cell sarcoma, recent molecular and cytogenetic findings, prognosis, and current approaches to treatment.[ncbi.nlm.nih.gov]
  • The nature of the disease and its relation to other diseases, treatment, prognosis and immunochemistry findings are being actively studied.[ncbi.nlm.nih.gov]

Etiology

  • [Article in English, Portuguese] Author information 1 Universidade Federal do Amazonas, Manaus, AM, Brasil. f.l.westphal@uol.com.br Abstract Castleman's disease is an atypical lymphoproliferative disorder of unknown etiology, which might be associated[ncbi.nlm.nih.gov]
  • The etiology, pathogenesis, optimal treatment, and prognosis are not well understood. In this review, we investigated these features to deepen the understanding of the disease.[ncbi.nlm.nih.gov]
  • TERMINOLOGY Abbreviations Follicular dendritic cell (FDC) sarcoma Synonyms FDC tumor Dendritic reticulum cell sarcoma Definitions Neoplastic proliferation of follicular dendritic cells Immunophenotype supports FDC lineage ETIOLOGY/PATHOGENESIS Normal[basicmedicalkey.com]
  • Monteverde, Marco Taurchini, William Grossi, Alessandra Dubini and Davide Dell'amore World Journal of Surgical Medical and Radiation Oncology 2013, 2:1 Abstract Follicular Dendritic Cell Sarcoma (FDCS) is an extremely rare malignant tumour of unknown etiology[npplweb.com]
  • Etiology FDC express CD21 which is EBV receptor, however the correlation is not well documented Hyaline-vascular Castleman disease may be a predisposing factor to FDC p53 pathway may play a role Epidermal growth factor receptor(EGFR) expression has[slideshare.net]

Epidemiology

  • […] testing Endoscopy Flow cytometry Immunophenotyping Liver function test Neurological exam Ultrasound exam Urinalysis Water deprivation test Progression and Transformation Local recurrences occur in more than 50% of case s Metastases occurs in 25% of case s Epidemiology[seer.cancer.gov]
  • Epidemiology 80 cases was documented until 2011 in English literature Pancreat Dis Int,Vol 10,No 4 • August 15,2011 Hepatobiliary Onset age is various (9-82), mean age is about 50 Female: male: 1.2:1 Presentation: Painless, slow-growing mass Lab:[slideshare.net]
  • […] disease (CD) Hyaline-vascular variant FDC “dysplasia” has been reported in hyaline-vascular CD Inflammatory pseudotumor-like variant of FDC sarcoma Consistently associated with Epstein-Barr virus (EBV) EBV is present in monoclonal form CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Epidemiology Only 56 confirmed cases have been reported in the English language literature. 24 of these were extra-nodal.[e-immunohistochemistry.info]
  • Epidemiology Follicular dendritic cell sarcomas are extremely rare. The number of reported cases are fewer than 100. Patients as young as 9 up to 82 years old have been described, though it occurs predominantly in adults.[atlasgeneticsoncology.org]
Sex distribution
Age distribution

Pathophysiology

  • This genomic characterization provides new information regarding follicular dendritic cell sarcoma biology that may improve understanding about the underlying pathophysiology, provide better prognostication, and identify potential therapeutic markers[ncbi.nlm.nih.gov]
  • A potential unifying pathophysiology could involve the elevation of interleukin-6, which has been previously described to contribute to lymphoproliferation associated with cases of CD and CD with MG.[neurology.org]
  • Spontaneous regression has been reported, perhaps suggesting an incomplete understanding of the pathophysiology 26. A number of factors could be responsible for the worse prognosis in intraabdominal, extranodal fdcs.[current-oncology.com]
  • Pharmacotherapy: a pathophysiologic approach. 2nd ed. Norwalk, Connecticut, USA: Appleton & Lange, 1993: 1879–929 Google Scholar 4. Waterhouse DN, Tardi PG, Mayer LD, et al.[doi.org]

Prevention

  • Resected localized disease may be prevented from recurrence by consolidative radiotherapy. Chemotherapy regimens have shown nondurable antitumor activity in FDCS. Patients with IDDCS usually present with lymphadenopathy.[ncbi.nlm.nih.gov]
  • You must be able to prove that the condition prevents you from performing any type of work activity.[disabilitybenefitscenter.org]
  • Because this significant recurrent and metastatic potential it is reasonable that resected localized disease may be prevented from recurrence by adjuvant radiotherapy or chemotherapy [ 11 – 13 ].[doi.org]
  • FDC sarcoma has numerous distinctive histological features that should serve as clues to bring the neoplasm into the differential diagnosis and thus prevent misdiagnosis.[ccij-online.org]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!